Iranian Journal of Pediatrics
Year:2006 | Volume:16 | Issue:3|Papers Count:22

Background: Myelomeningocele is the most common central nervous system birth defect compatible with life. The various impairments occurring in these children are skeletal, genitourinary, psychological defect and infections. The learning disabilities are usually overlooked. We conducted this study to evaluate the intelligence quotient (IQ) in these children using Ravens test.Methods: A case-control study was conducted in the Children's Medical Center in Tehran from February 2005 to 2006. The case group included children of 5-12 years old, with myelomeningocele that were admitted or referred due to complications or follow-up. The control group was selected from children that were referred to the hospital for reasons other than myelomeningocele and diseases affecting brain development or neurological status and were matched for age and sex. An IQ test (Ravens progressive matrices test) was taken and the rest of the data was collected by filling a questionnaire. The test was performed by the same person in the same circumstances for both groups.Findings: There were 47 cases for which 47 controls were randomly selected. The two groups were similar according to the socioeconomic status and the education level of the parents (p=0.59). The age of the patients at the time of surgery varied from 21 days to 8 years (mean=3.3 months). Only 21.3% of meningomylocele patients were ambulant with a normal gait. 46.8% of total patients had a ventriculoperitoneal shunt inserted and half of them had experienced shunt complications in the form of obstruction or infection. 72.3% of patients had incontinence (both for urine and feces). The IQ in the case group varied from 73 to 134 with a mean of 96.4, whereas it varied from 70 to 128 with a mean of 104.9% in the control group. A statistically significant correlation was found in comparing the IQ of both groups (p=0.000). In the case group none of the factors such as age at the time of meningomylocele repair, history of meningitis before repair, placement of shunt and/or its complications, urine and fecal incontinence, presence of urinary problems, usage of CIC, inability to have a normal ambulatory status, recurrent hospital admissions, orthopedic problems, had an effect on the IQ of the patients.Conclusions: Inspite that, the IQ of the case group was significantly lower than the control group, still 90% of the patients in the case group had an average IQ.

Background: Creatin Kinase (CK) is an enzyme that participates in the usage and storage of energy cycle in tissue especially the muscles. Elevation of CK in the serum of the newborns is due to CK-MB isoenzyme, which increases in skeletal muscles of the fetus. Muscle activity has an important role in the elevation of creatin kinase level, and there is a direct relationship between muscular activity and CK level, since vaginal delivery leads to increased pressure on skeletal muscles of the newborns this study was done to determine CK changes among newborns delivered by vaginal and Cesarean section.Methods: 180 term neonates who were born in Al-Zahra Hospital of Tabriz University of Medical Sciences from March to August 2005 were chosen. This selection was done in such a manner that we could divide cases into three groups of 60 neonates upon their method of delivery including vaginal, elective and urgent Cesarean Section, and every group was divided in two subgroups upon their Apgar score as being low or high each containing 30 newborns. One hour after delivery 1 milliliter blood was taken from the umbilical cord of neonates and titers of CK were analyzed by spectrophotometer method.Findings: There was a meaningful difference between the mean level of Creatin Kinase in the group of neonates with low Apgar score in comparison with high Apgar score (P=0.001). Also the mean level of CK was higher in neonates delivered via elective Cesarean (P=0.002) and this difference was meaningful in Cesarean section with labor pain (P=0.001) in comparison with vaginal delivery. As a whole there was a meaningful difference between Cesarean section and vaginal delivery (P=0.001), but no difference was found between elective and Cesarean section with labor pain (P=0.1).Conclusions: This study shows that the level of CK is higher in neonates with low Apgar score regardless of the method of delivery and probably this finding is the results of brain, cardiac and muscles hypoxia of the newborns. Also total CK is elevated in neonates born via Cesarean section (elective and urgent), this alteration may be the result of anesthesia and or mother tissue injury by surgerical procedure and that was transferred to the neonates.

Background: Epidemiology studies show a strong relationship between glycemia and diabetic complications. In order to achieve a long term near normal glucose control, intensive insulin therapy is required. To reach this goal, patients should be guided by frequent self monitoring of blood glucose and measurement of HbAc1 three to four times per year. This study was designed in order to find the tools to achieve metabolic control in type I diabetic patients.Methods: Medical records of 145 diabetic patients were evaluated and compared with world wide studies.Findings: Among 145 subjects 11.7% were received intensive insulin therapy, 29.6% of them had a glucometer for frequent self monitoring of blood glucose and HbA1c was regularly determined in 33% of patients. Among the studied subjects, 48.5% of them had some form of dyslipidemia and 19.5% developed microalbuminuria.Conclusions: Our findings indicated that our patients with type I diabetes are far from standard insulin therapy in comparison with other world wide studies (p<0.001). We need to develop alternative and simple strategies to improve the outcome. Education programs for diabetic patients and facilities such as complete insurance coverage for providing the best route for insulin therapy and monitoring of the patients are recommended.

Background: For patients who have renal involvement during urinary infection or children with Vesico-ureteral Reflux (VUR), renal Dimercaptosuccinic Acid (DMSA) scan is performed which exposes children to significant radiation. β²MG is a low molecular weight protein freely filtered by the glomeruli and then actively reabsorbed normally up to 99.9% in the proximal tubules; its urinary measurement is a good index of proximal tubular function of these cells as a primary screening test.Methods: Urinary ß²MG/Cr was measured in random urine samples in 53 pyelonephritis patients. Urine samples were obtained at the time of Voiding Cysto-Ureterography (VCUG). DMSA renal scan was performed in all patients. ß²MG and urine creatinine were tested by ELISA and auto analyzer, respectively.Findings: Twenty children had various grades of renal scar. Results were compared with ratios of 19 children with low uptake scanning and with 14 normal scanning. The mean urinary ß²MG/Cr was higher in the scarring group (5.23±10.6) than in the normal group (0.19±0.2) and in low uptake group (0.49±0.86). Patients with grade 3 had higher values (14.69± 15.82) than grades 1 (0.36± 0.35) and 2 (3.37± 5.2). Patients without renal scar had ß²MG/Cr ratio below 0.46 microgram/mg. The mean β2MG was higher in the VUR group (3/45± 7.97) than non-VUR group (0.23 ± 0.24) ug/mgCr (P= 0.01). The mean β2MG/Cr was higher in patients with VUR grades 4 and 5 (8.93±12.01) than patients with VUR grades 1 to 3 (0.81± 3.04) (P= 0.02).Conclusion: This study revealed that the maximum B2MG/Cr value in non-VUR children was 0.85 microgram/mgCr, higher values can be considered as a cut off point in screening children for VUR. Measurement of Urinary B2MG may be useful in the early detection of tubular damage in VUR patients and patients with renal scars.

Background: measurement of the blood pressure in neonates isn’t always easy and feasible by conventional method, although it is important and should be done by easy and precise method.Methods: we measured the blood pressure of 40 neonates that were admitted at the neonatal phototherapy ward of Namazee hospital. The systolic Blood pressure of each baby was evaluated with pulse detecting method and pulse Oximetry during inflation of cuff while the neonate was quiet or asleep (by two people separately).Findings: There was significant correlation between the measured blood pressure by plethysmography waveform of pulse oximetry and pulse detecting method. The mean difference of BP by Puls oximetry and Pulse method was 9 mmHg and in 86-92% of neonates the measured BP by pulse had only 10 mmHg difference with the blood pressure by pulse oximery.Conclusions: This study showed that measuring of blood pressure by pulse oximetry can be applied in neonatal population and the measured blood pressure is 9 mm Hg higher than pulse method.

Background: Cholestasis in infants has many causes. Idiopathic neonatal hepatitis and biliary atresia are the most common causes. The clinical presentations of cholestasis in infancy caused by neonatal hepatitis and biliary atresia are very similar. Diagnosis may be difficult on many occasions. The surgical treatment for biliary atresia should be performed as early as possible. Some of the other causes that need urgent treatment are galactosemia, sepsis and hypothyroidism.Methods: In this descriptive cross sectional study we reviewed the 203 patients with cholestasis. In all of them TORCH and metabolic disease were investigated and sonography, PIPIDA scan and liver biopsy were done.Findings: Idiopathic neonatal hepatitis (46.3%) and biliary atresia (30.5%), were the two main causes of cholestasis. After these metabolic diseases (13.7%) were in the third position.Conclusion: The approach to different causes of cholestasis should first be in the differentiation between idiopathic neonatal hepatitis and biliary atresia. After that treatable causes (metabolic, sepsis) must be considered.

Background: Neural tube defects (NTD) are a group of congenital malformations that include spina bifida, anencephaly and encephalocele. Some investigations have indicated that Zinc deficiency is one of the causative factors of NTDs. This study was done to determine the relation between neural tube defect and neonatal serum Zinc level in Gorgan - North of Iran.Methods: This case-control study was carried out on 23 newborns with NTD and 35 healthy newborns, in Dezyani hospital in Gorgannorth of Iran. Serum zinc level was assessed with spectrophotometery. Data was analyzed by SPSS soft ware. Findings: Mean of serum Zinc levels in case and control groups were 8 (±3.9) μmol/Lit and 12.3 (±4) μ Mol/Lit, respectively (p<0.001). Zinc deficiency was found in 43.5% of the cases and 8.6% of the controls (χ2 =9.73, p=0.002). The logistic regression analysis has shown an association between the presence of NTDs and zinc deficiency (OR=8.2, 95%CI: 1.9-34.7).Conclusion: This study showed that Zinc deficiency was thought to be one of the most important factors in the NTDs etiology in this area.

Background: Evaluation is a systematic process for collecting, analyzing and interpretation of the obtained information for the purpose of investigating how many the objectives in mind can be achieved. Education in an academic educational system means bringing about proper and increasing changes that affect the outcome of this system, that is affecting the students, and the aim of such evaluation was promotion of the quality of an education process.Methods: In this study, Department of Pediatrics Faculty of Medicine, Tehran University of Medical Sciences Tehran University of Medical Sciences, benefiting from opinion poll of university lectures, student and graduates have evaluated their system of educational in the years of 2003- 2004 and their aim was to remodel and adjust educational system with the society’s needs and promotion of the quality of such educations. The employed method was cross sectional and descriptive and analytical on the basis of 10 procedural steps and with in 8 sections dealing with evaluated factors. Such as faculty board members, management and organizational capability, students, manpower and logistic affairs, educational environments research work centers, health and therapeutic sections, educational equipment, research equipment, laboratory and diagnosis centers, educational courses and programs, teaching and learning process as well as satisfaction expressed by students.Findings: the results of the research showed that general average of 8 investigating sections in 2003 and 11 investigating sections in 2004, based on SWOTs model were respectively %53.3, %75.3 that may well be interpreted as a desirable research work. So 80% graduates of this group were satisfaction from their educational programs.Conclusion: Internal evaluation is the best indicator showing how much we should go to achieve certain aims, analyzes quality of the activity if such a system and by which we achieve logical and routine results.

Background: Congenital malformations are one of the most important problems in pediatrics. The estimation of the prevalence of malformations and some probable determinants were the purpose of this study.Methods: In this retrospective study, all of the newborns that were born during three years (2002-4) were included. Hospital files of 3840 newborns were studied retrospectively and the data were collected in checklist.Finding: 118 cases had at least a major or minor malformation. Over all the prevalence of malformations was 3.1%. Male newborns showed a higher prevalence of malformations than females but with no statistical significance. The skeletal system had the highest rate of malformations, while the genitourinary system and the head and neck deformities were in the second and third position. There were no significant relations between the prevalence of malformations and the maternal age, the height and weight of the newborns and the season of birth.Conclusion: The prevalence of malformations in this study was similar to previous studies.

Background: Voiding dysfunction is a problem due to mal toilet training in urination. The prevalence is approximately 10-15%. The clinical manifestations are frequency, dysuria, incontinence, enuresis and infrequency with constipation.Methods: In this study in 70 children aged 3-12 years old with urinary symptoms of voiding dysfunction were studied the Dysfunctional Voiding Scoring System (DVSS) chart was filled. After one to three months of treatment for the urinary and gastroenterological symptoms, the results of acquired scored before and after treatment were compared.Findings: Among 70 children 78.6% were female and 21.4% were male. 45.7% were neurotic, 61.4% had a history of urinary tract infection and 75.7% had chronic constipation. The acquired score after treatment was significantly better than before treatment (P= 0.000).Conclusions: DVSS is a practical method for diagnosis, grading and evaluation of voiding dysfunction. It seems logical that the first step for management of V.D is medical therapy and urodynamic studies should be restricted to resistant or recurrent cases.

Background: The purpose of this study was to compare the efficacy and side effects of Filgrastim (Granulocyte, Colony Stimulating Factor [G-CSF] made by Roche, Swiss) and PD-grastim (An G-CSF Analogous made by Pooyesh Daru, IRAN) on chemotherapy related neutropenia in neuroblastoma patients.Methods: This randomized clinical trial was performed on children with Neuroblastoma. The inclusion criteria were: histopathologic proven neuroblastamo, age<16 years, Stage 3 or 4, without fever and infection, Absolute neutrophil count more than 1000/μl and platelet more than 100,000/μl and OPEC chemotherapy protocol which consists of (Oncovin, Platinium, Etoposide and Cyclophosphamide). This protocol was used every 28 days for 12 courses, in each course after chemotherapy 10μg/kg G-CSF (Filgrastim or PD-grastim) was used for 4 days. Patients were assigned randomly as odd and even numbers. Odd number patients received PD-grastim in odd courses & Filgrastim in even courses and vice versa in even number patients. A cell blood count was taken in the beginning and in the 6, 9, 13 days of the treatment, then two times weekly until neutrophil count reached 1000 μl/dl.Findings: Eight Neuroblastoma (6 males and 2 females) with mean Age 2.5 years were studied. Two cases were new and others were in the middle of their chemotherapy courses. Forty four cycles (Chemotherapy plus G-CSF) were studied which in 22 cycles Filgrastim and in 22 cycles PD-grastim were used. Neutropenia was found in 34 cycles. The mean time of neutropenia in Filgrastim group was 6.4±4.68 days and in PD-grastim group was 6.5±5.09 days. Among the 2 groups there was no significant difference in the incidence, intensity, and duration of neutropenia. All patients were discharged after 9 days without any complications or side effect.Conclusion: This study shows that there are no significant differences in the efficacy and side effects of Filgrastim versus PDgrastim in prevention of neutropenia after OPEC protocol chemotherapy treatment in stage 3& 4 neuroblastoma patients.

Background: The aim of the present study was to assess the effect of an ergo metric training program on the pregnancy outcome.Methods: Samples (90 pregnant) females were randomly selected in two experimental (No: 40) and control (No: 50) groups. In the experimental group, a training program for a period of 8 weeks (two sessions each week; each session consisted of 15-30 min) with an intensity of 50-60% of maximal heart rate was performed.Findings: Results showed no significant difference in pregnancy length, near birth neonate weight, 1st min APGAR, mechanical amniotic fluid and the type of feeding between experimental and control groups (p>0.05). But the comparison of 5th min APGAR and the type of delivery in both experimental and control groups showed a significant difference (P<0.05).Conclusion: It was concluded that a suitable physical activity in pregnancy not only causes no injury to the mother, but it guaranties a safer delivery and a sound birth.

Background: Simultaneous immunization against diphtheria, tetanus and pertussis during infancy and childhood has been a routine practice in Iran .Considering the role of triple vaccine in reduction of mortality as a result of disastrous diseases such as diphtheria, tetanus, and pertussis. We have little information on the incidence of its complications in Yazd. This study was performed to determine the complications of triple vaccine in the health centers of Yazd in 2005.Methods: The descriptive strategy of this study was carried out on 474 cases from 1.5 month to 6 years old. All of them were carefully examined up to 72h for the occurrence of complications and the observed symptoms were collected in a questionnaire and the data were analyzed.Findings: The incidence rate of complications after injection of triple vaccine was 81.4%. In this study fever, pain at the injection site, swelling, crying, loss of appetite, irritability, erythema, and vomiting were 56.8%, 55%, 26.8%, 11.4%, 11.2%, 11%, 10.5% and 6.5%, respectively.Conclusion: Due to the high incidence rate of complications after injection of triple vaccine, we recommend acetaminophen prophylacticly after vaccination.

Background: Attempt of suicide in children is somewhat different than with adults and little is known about childhood suicide and it’s responsible factors in children. In this study suicide in children under 14 years old and its etiologic factors have been reviewed.Methods: In a period of 3 years(since April 2002 to April 2004) among 176 children (8-13 years old) who were admitted for poisoning in Loghman university hospital, 111 patients (59 boys and 52 girls) that had an attempt of suicide were studied. Demographic data, living area, drugs used, time and place of suicide, past history of psychologic and somatic disorders and psychologic consultation data were gathered and analyzed.Findings: Based on this study in children 8-13 years old, poisoning was intentional in 63% of cases. Attempt of suicide was a little more common in boys, most of the cases were between 12-13 years old and most of them lived in a low socioeconomic area (57.4%). Suicide was often attempted at home (95.5%) and cases frequently occurred in the winter (35.6%). Benzodiazepines (1 9.3%) followed by tricyclic antidepressant (14%) and anticonvulsants (12%) were the most common drugs used. The drugs had often been available for the children (92.8%). Thirteen patients had previous history of attempt of suicide and 47% of patients had psychologic disorder.Conclusion: According to this study attempt of suicide was the most common cause of poisoning in children between 8-13 years old and socioeconomic status, background of psychological disorders and exposure to potentially toxic drugs made their attempt of suicide easier.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disease of normal but overactive histiocytes (macrophages) that commonly appears in childhood. A fever, hepatosplenomegaly, pancytopenia, lymphadenopathy, and a rash often comprise the initial presentation. Cutaneous involvement occurs in up to 65% of patients. Although the signs of central nervous system (CNS) involvement may be pronounced early, it usually develops later during the course of the disease. The pathological hallmark of this disease is the aggressive proliferation of activated macrophages and histiocytes, which phagocytose other cells, namely red blood cells, white blood cells, and platelets, leading to the clinical symptoms.Case Report: This paper presents a 4- years old girl that was admitted to the hospital because of abdominal distention .Her vital signs were normal. Physical examinations by her physician were revealed hepatosplenomegaly. She had pancytopenia, high erythrocyte sedimentation rate but tests for liver and kidney-functions were normal in primary laboratory data. The results of tests for viral hepatitis A, B, and C were negative. An abdominal ultrasonographic study confirmed the presence of hepatosplenomegaly, but no ascites was detected. Examination of a stained bone marrow aspirate (BMA) revealed dyserythropoietic change. After 2 months, she presented with intermittent fever but three blood cultures yielded no growth at 48 hours. A liver biopsy was done and it showed a picture similar to chronic persistent hepatitis. Again, she has undergone bone marrow aspiration and showed the same findings with the past BMA (dyserythropoietic change) .She was candidate for bone marrow biopsy and explorative laparotomy. During explorative laparotomy, a specimen from liver (again), spleen, pancreas, and abdominal lymph nodes was obtained. There was evidence of hemophagocytosis in the spleen, marrow, lymph nodes specimens and the results of complementary laboratories were consistent with a "Hemophagocytic Lymphohistiocytosis". 

Objective: Fraser syndrome is characterized by cryptophthalmos, cutaneous syndactyly, malformations of the larynx and genitourinary tract, craniofacial dysmorphism, orofacial clef ting, mental retardation, and musculoskeletal anomalies. The prevalence of FS was previously estimated to be approximately 11 cases in 100.000 live births. The inheritance is autosomal recessive. This is the first report of this syndrome in Iran.Case presentation: We present a 2 days old girl neonate with the chief complaint of unilateral cryptophthalmos (left side) and Fraser syndrome. The mains symptoms and signs of this patient were low set ears, abdominal distention and vomiting, naries closure, upper extremity, syndactyly and rectovestibular fistula. Sonography and ecchocardiography was done and a single right kidney in right upper quadrant and patent ductus arteriosus (PDA) were detected. The patient was operated with under total corrected anoplasty and was discharged with a good general condition.Conclusion: Fraser syndrome is a rare syndrome, but it must be suggested in craniofacial malformation especially cryptophthalmos.  

Objective: Treacher Collins syndrome or mandibulofacial dysostosis is a rare genetic syndrome characterized by a small mandible and ear anomalies. Some of the patients have congenital heart disease. Case report: A 10-months old female infant with micrognathia, bilateral conductive hearing loss needing hearing aid, and patent ductus arteriosus is presented. Chromosomal study yielded normal karyotype and Treacher Collins syndrome was the first diagnosed by the genetician. The ductus was occluded by a pfm coil.Conclusion: In general, patient affected with Treacher Collins syndrome have no mental or skeletal problems and they can be productive members of the society. So diagnosis and treatment of their congenital heart abnormalities has a greater importance in comparison with patients affected by other genetic syndromes.

Consanguineous marriage is common in our country, accounting for more than 35% of marriages in Iran. The complications behind this practice are clear in the medical genetics. This paper tries to clarify the importance of this subject and find some answers to questions faced by medical professionals especially pediatricians. Premarital genetic counseling is the best and appropriate method to screen any inherited disorder in the families.